Cargando…

Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis

Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular sp...

Descripción completa

Detalles Bibliográficos
Autores principales: Obiorah, Ifeyinwa Emmanuela, Ozdemirli, Metin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5949169/
https://www.ncbi.nlm.nih.gov/pubmed/29854538
http://dx.doi.org/10.1155/2018/5364985
_version_ 1783322693749178368
author Obiorah, Ifeyinwa Emmanuela
Ozdemirli, Metin
author_facet Obiorah, Ifeyinwa Emmanuela
Ozdemirli, Metin
author_sort Obiorah, Ifeyinwa Emmanuela
collection PubMed
description Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow. Lymph nodes are typically spared. The clinical picture depends on the specific organ involvement making the correct diagnosis very difficult. Here, we report a case of intravascular large B-cell lymphoma diagnosed postmortem on a 69-year-old African-American male who presented with unilateral proptosis and visual loss. An initial diagnosis of temporal arteritis was made and the patient received corticosteroids. However, the patient developed multiorgan failure and expired. On autopsy, there was disseminated intravascular lymphoma involving predominantly vessels within the heart, kidneys, liver, stomach, lungs, adrenal glands, small intestine, bladder, thyroid, and brain. Interestingly, there was also partial involvement of the retroperitoneal lymph nodes which is an unusual presentation in this disorder. Immunohistochemical staining showed that the lymphoma cells were positive for CD20, indicating B-cell phenotype. This case supports the “mimicking nature” of this rare entity with an unusual presentation with proptosis and visual loss, simulating temporal arteritis and a rare involvement of the retroperitoneal lymph nodes. The presentation of intravascular large B-cell lymphoma can vary, and the key to diagnosis is dependent on histopathology and immunohistochemistry. Increased awareness, early tissue diagnosis, and prompt chemotherapy are crucial for this otherwise lethal disease.
format Online
Article
Text
id pubmed-5949169
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher Hindawi
record_format MEDLINE/PubMed
spelling pubmed-59491692018-05-31 Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis Obiorah, Ifeyinwa Emmanuela Ozdemirli, Metin Case Rep Rheumatol Case Report Intravascular lymphoma is a rare type of lymphoma, characterized by growth of lymphoma cells within the microvasculature. The majority of the cases are of B-cell lineage, although rare examples of T or NK lineage have also been reported. The lymphoma is usually widely disseminated in the vascular spaces of any organ at the time of diagnosis including the skin and bone marrow. Lymph nodes are typically spared. The clinical picture depends on the specific organ involvement making the correct diagnosis very difficult. Here, we report a case of intravascular large B-cell lymphoma diagnosed postmortem on a 69-year-old African-American male who presented with unilateral proptosis and visual loss. An initial diagnosis of temporal arteritis was made and the patient received corticosteroids. However, the patient developed multiorgan failure and expired. On autopsy, there was disseminated intravascular lymphoma involving predominantly vessels within the heart, kidneys, liver, stomach, lungs, adrenal glands, small intestine, bladder, thyroid, and brain. Interestingly, there was also partial involvement of the retroperitoneal lymph nodes which is an unusual presentation in this disorder. Immunohistochemical staining showed that the lymphoma cells were positive for CD20, indicating B-cell phenotype. This case supports the “mimicking nature” of this rare entity with an unusual presentation with proptosis and visual loss, simulating temporal arteritis and a rare involvement of the retroperitoneal lymph nodes. The presentation of intravascular large B-cell lymphoma can vary, and the key to diagnosis is dependent on histopathology and immunohistochemistry. Increased awareness, early tissue diagnosis, and prompt chemotherapy are crucial for this otherwise lethal disease. Hindawi 2018-04-29 /pmc/articles/PMC5949169/ /pubmed/29854538 http://dx.doi.org/10.1155/2018/5364985 Text en Copyright © 2018 Ifeyinwa Emmanuela Obiorah and Metin Ozdemirli. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Obiorah, Ifeyinwa Emmanuela
Ozdemirli, Metin
Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title_full Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title_fullStr Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title_full_unstemmed Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title_short Intravascular Large B-Cell Lymphoma Mimicking Temporal Arteritis
title_sort intravascular large b-cell lymphoma mimicking temporal arteritis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5949169/
https://www.ncbi.nlm.nih.gov/pubmed/29854538
http://dx.doi.org/10.1155/2018/5364985
work_keys_str_mv AT obiorahifeyinwaemmanuela intravascularlargebcelllymphomamimickingtemporalarteritis
AT ozdemirlimetin intravascularlargebcelllymphomamimickingtemporalarteritis